Primary nephrotic syndrome (NS) is the most common glomerular disease in children. Therapeutic options for frequent relapser and steroid-dependent patients frequently include long lasting treatment with prednisone and calcineurin antagonists. Immunoglobulins are widely used in autoimmune diseases.

Sixty patients were analyzed (male=61.7%, median age at diagnosis 3.5 years (2.5; 5.0)). All were steroid-dependent. The median time from diagnosis to initiation of the 3 treatments was 2.3 years (0.7; 3.0). Previous treatment included calcineurin inhibitors (15 patients), Rituximab (9 patients), MMF (18 patients), levamisole (22 patients) and cyclophosphamide (3 patients). The median duration of treatment was 6.3 months (5.0; 7.3). Two patients didn’t complete the treatment because of bad tolerance of the weekly subcutaneous injections. Steroids were discontinued in every patient but 1. Eight patients were receiving tacrolimus at onset and tacrolimus was discontinued in all 8 patients. Thirty-eight patients (63.3%) relapsed, 24 of them without any ongoing treatment and 14 of them with at least 1 treatment. Median time to relapse after the beginning of the association was 10.6 months (7.8-14.4). Twenty patients (33.3%) are free of relapse after a median time of 16.8 months (14.6-23.7) after treatment withdrawal. None of the patients experienced any serious adverse effect.

Conclusions:

The association of MMF, levamisole and SCIg, was given to optimize the withdrawal of steroids and calcineurin inhibitor in patients with idiopathic steroid-dependent NS. Interestingly 33.3% patients remained free of relapse 16.8 months after withdrawal of all treatments.